Impact of liver-specific survival motor neuron (SMN) depletion on central nervous system and peripheral tissue pathology
Published: Feb. 10, 2025
Spinal
muscular
atrophy
(SMA)
is
an
inherited
neuromuscular
disorder
stemming
from
deletions
or
mutations
in
the
Survival
Motor
Neuron
1
(
SMN1
)
gene,
leading
to
decreased
levels
of
SMN
protein,
and
subsequent
motor
neuron
death
muscle
atrophy.
While
traditionally
viewed
as
a
predominantly
affecting
neurons,
recent
research
suggests
involvement
various
peripheral
organs
SMA
pathology.
Notably,
liver
has
emerged
significant
focus
due
observed
fatty
phenotype
dysfunction
both
mouse
models
patients.
Despite
these
findings,
it
remains
unclear
whether
intrinsic
depletion
protein
contributes
pathology
central
nervous
systems.
To
address
this
knowledge
gap,
we
developed
model
with
liver-specific
by
utilizing
Alb-Cre
transgene
together
one
Smn
2B
allele
exon
7
flanked
loxP
sites.
Initially,
evaluated
phenotypic
changes
mice
at
postnatal
day
19
(P19),
time
when
severe
SMA,
2B/-
mice,
typically
exhibit
many
symptoms
disease.
Our
findings
indicate
that
does
not
induce
death,
atrophy,
characteristics
P19.
However,
mild
steatosis
was
point,
although
no
function
were
detected.
pancreatic
alterations
resembled
decrease
insulin-producing
β-cells
increase
glucagon-producing
α-cells,
accompanied
reduction
blood
glucose
plasma
glucagon
glucagon-like
peptide
(GLP-1)
levels.
Moreover,
transient,
P60
exhibited
recovery
function.
mosaic
pattern
Cre-mediated
excision
precludes
definitive
conclusions
regarding
contribution
overall
tissue
pathology,
our
highlight
intricate
connection
between
abnormalities
adding
nuanced
layer
understanding
disease’s
complexities.
Language: Английский
Impact of liver-specific survival motor neuron (SMN) depletion on central nervous system and peripheral tissue pathology
eLife,
Journal Year:
2025,
Volume and Issue:
13
Published: Feb. 20, 2025
Spinal
muscular
atrophy
(SMA)
is
caused
by
mutations
in
the
Survival
Motor
Neuron
1
(
SMN1
)
gene.
While
traditionally
viewed
as
a
motor
neuron
disorder,
there
involvement
of
various
peripheral
organs
SMA.
Notably,
fatty
liver
has
been
observed
SMA
mouse
models
and
patients.
Nevertheless,
it
remains
unclear
whether
intrinsic
depletion
SMN
protein
contributes
to
pathology
or
central
nervous
systems.
To
address
this,
we
developed
model
with
liver-specific
utilizing
an
Alb-Cre
transgene
together
one
Smn
2B
allele
Smn1
exon
7
flanked
loxP
sites.
Initially,
evaluated
phenotypic
changes
these
mice
at
postnatal
day
19
(P19),
when
severe
SMA,
2B/-
mice,
exhibit
many
symptoms
disease.
The
does
not
induce
death,
neuromuscular
muscle
atrophy,
characteristics
typically
P19.
However,
mild
steatosis
was
observed,
although
no
function
were
detected.
pancreatic
alterations
resembled
that
decrease
insulin-producing
β-cells
increase
glucagon-producingα-cells,
accompanied
reduction
blood
glucose
plasma
glucagon
glucagon-like
peptide
(GLP-1).
These
transient,
P60
exhibited
recovery
function.
mosaic
pattern
Cre-mediated
excision
precludes
definitive
conclusions
regarding
contribution
overall
tissue
pathology,
our
findings
highlight
intricate
connection
between
abnormalities
Language: Английский
Experimental and interaction mechanism with novel depressant lysine for the removal of copper ions activation on sphalerite surface
Lingyun Bao,
No information about this author
Wengang Liu,
No information about this author
Wenbao Liu
No information about this author
et al.
Minerals Engineering,
Journal Year:
2025,
Volume and Issue:
226, P. 109247 - 109247
Published: March 14, 2025
Language: Английский
Multicomponent Metabiotic Actoflor-S Has Therapeutic Potential for Bowel, Liver, and Metabolic Disorders
IntechOpen eBooks,
Journal Year:
2025,
Volume and Issue:
unknown
Published: April 10, 2025
One
of
the
aspects
bacterial
symbiosis
with
host
is
exchange
metabolites.
Stimulation
growth
beneficial
bacteria
in
microbiota
using
metabiotic
Actoflor®-S
led
to
a
change
composition
flow
its
Analysis
blood
metabolome
mice
by
GC-MS
showed
that
animals
receiving
had
an
increased
concentration
almost
all
amino
acids
and
decreased
most
carboxylic
their
derivatives,
including
lactic
acid.
Comparison
obtained
results
literature
data
action
leads
normalization
at
least
10
marker
compounds,
whose
was
significantly
changed
patients
ulcerative
colitis.
The
property
normalize
turned
out
be
inherent
only
entire
set
components,
but
not
parts,
which
indicator
emergent
properties
metabiotic.
Overall,
work
also
indicate
metabolites
are
important
participants
maintaining
health;
often,
they
have
individual
activity,
cases,
activity
consequence
combined
action.
Language: Английский
Alterations in hepatic amino acid metabolism related to MASLD in individuals with obesity
Journal of Physiology and Biochemistry,
Journal Year:
2025,
Volume and Issue:
unknown
Published: May 8, 2025
Language: Английский
Metabolomics at the cutting edge of risk prediction of MASLD
En Ying Tan,
No information about this author
Mark Muthiah,
No information about this author
Arun J. Sanyal
No information about this author
et al.
Cell Reports Medicine,
Journal Year:
2024,
Volume and Issue:
unknown, P. 101853 - 101853
Published: Dec. 1, 2024
Language: Английский
Impact of liver specific survival motor neuron (SMN) depletion on peripheral and central nervous system tissue pathology
eLife,
Journal Year:
2024,
Volume and Issue:
13
Published: Sept. 24, 2024
Spinal
muscular
atrophy
(SMA)
is
caused
by
mutations
in
the
Survival
Motor
Neuron
1
(SMN1)
gene.
While
traditionally
viewed
as
a
motor
neuron
disorder,
there
involvement
of
various
peripheral
organs
SMA.
Notably,
fatty
liver
has
been
observed
SMA
mouse
models
and
patients.
Nevertheless,
it
remains
unclear
whether
intrinsic
depletion
SMN
protein
contributes
to
pathology
or
central
nervous
systems.
To
address
this,
we
developed
model
with
liver-specific
utilizing
an
Alb-Cre
transgene
together
one
Smn2B
allele
Smn1
exon
7
flanked
loxP
sites.
Initially,
evaluated
phenotypic
changes
these
mice
at
postnatal
day
19
(P19),
when
severe
SMA,
Smn2B/-
mice,
exhibit
many
symptoms
disease.
The
does
not
induce
death,
neuromuscular
muscle
atrophy,
characteristics
typically
P19.
However,
mild
steatosis
was
observed,
although
no
function
were
detected.
pancreatic
alterations
resembled
that
Smn2B/-mice,
decrease
insulin-producing
β-cells
increase
glucagon-producingα-cells,
accompanied
reduction
blood
glucose
plasma
glucagon
glucagon-like
peptide
(GLP-1).
These
transient,
P60
exhibited
recovery
function.
mosaic
pattern
Cre-mediated
excision
precludes
definitive
conclusions
regarding
contribution
overall
tissue
pathology,
our
findings
highlight
intricate
connection
between
abnormalities
Language: Английский
The toxicity of cisplatin derives from effects on renal organic ion transporters expression and serum endogenous substance levels
Mingkang Zhang,
No information about this author
Yile Li,
No information about this author
Yanrong Ma
No information about this author
et al.
Food and Chemical Toxicology,
Journal Year:
2024,
Volume and Issue:
192, P. 114949 - 114949
Published: Aug. 24, 2024
Language: Английский
UHPLC-QQQ-MS/MS method for the simultaneous quantification of 18 amino acids in various meats
Frontiers in Nutrition,
Journal Year:
2024,
Volume and Issue:
11
Published: Oct. 30, 2024
Amino
acids
are
an
essential
source
of
human
protein,
and
their
content
composition
the
main
factors
determining
food
protein
utilization
rate.
Determining
amino
is
in
component
analysis
food.
Therefore,
a
groundbreaking
technique
was
developed
utilizing
ultra-high
performance
liquid
chromatography
interfaced
with
triple
quadrupole
mass
spectrometer
(UHPLC-QQQ-MS/MS)
for
concurrently
quantifying
18
across
various
types
meat.
According
to
test
results,
it
can
be
known
that
average
glutamate
(2.03
×
10
Language: Английский